Mechanical Ventilation for Duchenne Muscular Dystrophy: Sinner or Saint?

Smith, Barbara K.; Falk, Darin J.

doi:10.1002/mus.25986

Cited by 2 publications

(2 citation statements)

References 19 publications

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“…As DMD is progressive loss of muscle tissue, mechanical ventilation enables oxygenation in respiratory muscles weakness and restrictive breathing disorders. Most patients are mechanically ventilated at home through a tracheostomy tube [16,17]. Prolonged mechanical ventilation may cause insomnia, pain, daytime sleepiness and digestive problems, yet patients do not complain about their quality of life [18].…”

Section: Discussionmentioning

confidence: 99%

Aggravation of Cardiovascular and Respiratory Decline in Advanced Duchenne Muscular Dystrophy Complicated by Dilated Cardiomyopathy – Case Study and Review of Literature

Welian-Polus,

Mazur,

Bielak

et al. 2024

J Educ Health Sport

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Introduction: Duchenne muscular dystrophy is a genetic X-linked recessive disorder. This condition is characterized by progressive loss of muscle tissue. Thus, it results in deterioration and inability to perform basic motor skills such as independent movement or breathing. Due to progressive muscle weakness, patients with advanced stages of DMD require mechanical ventilation, feeding, and rehabilitation. Furthermore, alterations in cardiac muscle lead to cardiomyopathy. Despite advanced supportive treatment, DMD is a fatal disease. Purpose: The aim of the paper is to present, using a case study description, the current standards of treatment for patients with Duchenne muscular dystrophy as well as the current state of knowledge and new discoveries regarding this medical condition. Material and methods The patient's medical records were analyzed and available literature in PubMed was reviewed to write this article using the keywords: „Duchenne muscular dystrophy”; „cardiomyopathy”; „mechanical ventilation”; „gene therapy”; Conclusions Early detection of respiratory and circulatory insufficiency improves the patient's quality of life. Many patients with an advanced stage of Duchenne muscular dystrophy need specialized treatment, for example, in the intensive care unit. Therefore there is an urgent need for new treatment methods, such as gene therapies, which can slow down or break the course of the disease. New discoveries and the implementation of new treatment standards can enhance the quality of life for patients and extend their lifespans.

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Section: Discussionmentioning

confidence: 99%

Aggravation of Cardiovascular and Respiratory Decline in Advanced Duchenne Muscular Dystrophy Complicated by Dilated Cardiomyopathy – Case Study and Review of Literature

Welian-Polus,

Mazur,

Bielak

et al. 2024

J Educ Health Sport

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show abstract

“…MV is a life-saving supportive therapy for patients with acute respiratory failure, while it is often referred to as a two-edged sword for patients in cardiogenic shock [24]. MV has been associated with some major complications, such as infection, barotrauma, tracheal injury, and ventilator-induced lung injury [25], especially the ventilator-induced di culty in weaning [26].…”

Section: Discussionmentioning

confidence: 99%

Diaphragm Satellite Cells Isolation by Optimized MACS and the Effect of Mechanical Ventilation on their Proliferation and Differentiation Characteristics through MyoD and Myogenin Pathways

Ding

Liang

et al. 2020

Preprint

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Objective: In the present study, we aimed to establish a feasible method to isolate single diaphragm satellite cells from C57 mice, and clarify the effect of mechanical ventilation (MV) on the proliferation and differentiation of diaphragm satellite cells. Moreover, the underlying molecular mechanism was also explored.Methods: After the dissection of the diaphragm, enzymolysis, and specific antibody selection, single diaphragm satellite cells were harvested from C57 mice receiving 6 h of MV or not with optimized magnetic-activated cell sorting (MACS) approach. The cells were stained with BrdU or labeled with the differentiation antibody MYH3, followed by observation using fluorescence microscopy. The cells were counted from randomly selected visual fields, and the proliferation or differentiation characteristics of the control and MV groups were compared by IMAGE software. Besides, the expressions of MyoD and myogenin were detected by quantitative real-time PCR (qRT-PCR). Results: The single diaphragm satellite cells were successfully purified through MACS using a set of optimized parameters. Generally speaking, 1.5×105 cells could be harvested from a single diaphragm. Upon MV, the proliferation rate of diaphragm satellite cells was decreased from 88.74% to 81.92%, while the differentiation rate was increased from 17.94% to 27.58%. Moreover, the expressions of MyoD and myogenin were significantly up-regulated upon MV. Conclusions: In our current work, an efficient method was successfully established to isolate single diaphragm satellite cells. After MV, the differentiation rate of diaphragm satellite cells tended to increase, and the expressions of MyoD and myogenin were up-regulated. Collectively, our findings provided valuable insights into further research and clinical target treatment.

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Mechanical Ventilation for Duchenne Muscular Dystrophy: Sinner or Saint?

Cited by 2 publications

References 19 publications

Aggravation of Cardiovascular and Respiratory Decline in Advanced Duchenne Muscular Dystrophy Complicated by Dilated Cardiomyopathy – Case Study and Review of Literature

Aggravation of Cardiovascular and Respiratory Decline in Advanced Duchenne Muscular Dystrophy Complicated by Dilated Cardiomyopathy – Case Study and Review of Literature

Diaphragm Satellite Cells Isolation by Optimized MACS and the Effect of Mechanical Ventilation on their Proliferation and Differentiation Characteristics through MyoD and Myogenin Pathways

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